case report primary cardiac burkitt lymphoma presenting...

Case ReportPrimary Cardiac Burkitt Lymphoma Presenting withAbdominal Pain

Dimitrios Tzachanis,1 Rajan Dewar,2 Katarina Luptakova,2

James D. Chang,2 and Robin M. Joyce2

1 Cedars-Sinai Medical Center, 8700 Beverly Boulevard, AC1046-A, Los Angeles, CA 90048, USA2 Beth Israel Deaconess Medical Center, 330 Brookline Avenue, Boston, MA 02215, USA

Correspondence should be addressed to Dimitrios Tzachanis; [email protected]

Received 6 August 2014; Accepted 17 October 2014; Published 6 November 2014

Academic Editor: Marie-Christine Kyrtsonis

Copyright © 2014 Dimitrios Tzachanis et al. This is an open access article distributed under the Creative Commons AttributionLicense, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properlycited.

We describe the case of a 44-year-old woman with primary Burkitt lymphoma of the heart who presented with abdominal bloatingand epigastric discomfort secondary to tamponade physiology caused by a large pericardial effusion.The pericardial fluid containeda large number of highly atypical lymphocytes with moderate basophilic cytoplasm, rare punched-out vacuoles, a vesicular nuclearchromatin, large nucleolus, and marginated chromatin that by FISH were positive for the 8;14 translocation. She had no other sitesof disease. She was treated with four alternating cycles of modified CODOX-M and IVAC in combination with rituximab andremains in remission more than 5 years since diagnosis.

1. Background

Primary cardiac tumors are rare. Most of them are benignwith myxoma being the most frequent histologic type [1–3]. Primary malignant cardiac tumors are 100–1000 timesless common than metastatic disease to the heart. The mostfrequent primary malignant cardiac cancer is sarcoma. Lym-phomas are diagnosed in about 1-2% of patients with a pri-mary cardiac tumor and occur more frequently in immuno-suppressed hosts such as in patients infected with the humanimmunodeficiency virus (HIV) [4]. Lymphomas are thesecond most common tumors involving the heart in HIVinfected patients reflecting the higher incidence of lymphomaand of extranodal involvement in patients with HIV [5].

Diffuse large B cell lymphoma is the most commonhistologic type of cardiac lymphoma. Primary cardiac Burkittlymphoma (BL) has been reported very rarely. In our reviewof the literature we identified 4 cases in adults. Kuroda et al.reported the case of a 79-year-old female with cardiac BL inthe form of a left ventricular mass and a massive pericardialeffusion. Diagnosis was made by pericardiocentesis that

showed large B cells that had an t(8;22) translocation. Thepatient initially went into remission with chemotherapy withCVP (cyclophosphamide, vincristine, and prednisone) butrelapsed in the brain and eventually succumbed to her disease[6]. Fatimi et al. reported the case of an elderly man withprimary cardiac BL who was initially misdiagnosed as havingintracardiac thrombus and acute pulmonary embolism [7].Stefani et al. published the case of a 61-year-old woman withBL who presented with a large mass arising from the rightventricle andwith associated pericardial and pleural effusions[8]. The mass was almost completely excised but by the timeshe recovered from the surgery and started systemic treat-ment the lymphoma was disseminated and she died beforecompleting the first cycle of chemotherapy. Peng et al. pre-sented the case of a 33-year-old man with cardiac BL present-ing with a right atrial mass extending into the myocardiumand pericardium and with paratracheal lymphadenopathy[9].

In the literature we identified 3 more pediatric cases.Chalabreysse et al. in 2002 described a case of primary BL aspart of their review of the only 35 cases of primary cardiac

Hindawi Publishing CorporationCase Reports in HematologyVolume 2014, Article ID 687598, 4 pageshttp://dx.doi.org/10.1155/2014/687598

2 Case Reports in Hematology

Figure 1:The pericardial fluid was bloody. Within the erythrocytes,numerous atypical intermediate to large cells were seen.Many of thecells had a vesicular chromatin and 1–3 prominent nucleoli (whitearrows). Conspicuously, there were numerous apoptotic debris andhistiocytes with ingested debris (yellow arrows).

lymphoma published to that date [10]. It was the case ofa 9-year-old boy with three intracardiac masses and whowas treated successfully with surgical resection followed bymultiagent chemotherapy. Fresneau et al. reported the caseof a 17-year-old female with cardiac BL arising in the rightatrium and with cervical and mediastinal lymphadenopathy[11]. Meshref et al. describe the case of a 10-year-old boywith cardiac BL who presented with multiple intracardiacmasses [12]. Finally there are two reported cases of Africanor endemic BL with primary cardiac involvement [13, 14].

We herein report the case of a 44-year-old woman withprimary BL of the heart.This is only the fifth reported case inan immunocompetent adult.

2. Case

A previously healthy 44-year-old woman presented to theemergency room with early satiety, abdominal bloating, andepigastric discomfort that had started about a week ago. Aright upper quadrant ultrasound was done followed by a CTof the abdomen and pelvis. The CT scan showed a partiallyvisualized large pericardial effusion. There was no lym-phadenopathy. She then underwent an echocardiogram thatshowed a hyperdynamic left ventricle systolic function withlarge circumferential pericardial effusion with tamponadephysiology. She was taken to the cath lab where a pericardio-centesis was performed and a total of 730 cc of sanguineousfluid was drained and a pericardial drain was placed. Thepericardial fluid contained 65,000WBCs with the differentialshowing 6% polys, 1% lymphocytes, 1% monocyte, and92% highly atypical lymphocytes with moderate basophiliccytoplasm, rare punched-out vacuoles. Most of the largeatypical cells had a vesicular nuclear chromatin, some withlarge nucleolus and marginated chromatin. A hematoxylinand eosin (H&E) stained cell block preparation is shown inFigure 1. This in addition shows numerous apoptotic debrisand histiocytes with ingested debris. In tissue sections of BL,the histiocytes and apoptotic debris constitute the starry-sky appearance. Similar findings were seen in this cell blockpreparation, providing morphological evidence of a highly

RA

LA

LV

RV

Figure 2: Steady-state free precession magnetic resonance imagedemonstrating a broad based, isointense structure (black arrow)measuring 3 cm × 1 cm arising from the lateral wall of the rightatrium adjacent to the atrioventricular junction of the right atrium.Connected to this broad based mass is an isodense, circular,well-circumscribed mass (red arrow) measuring 1 cm in diameterprotruding from the broad based mass into the right atrium. RA:right atrium, RV: right ventricle, LA: left atrium, LV: left ventricle.

proliferative lymphoma. Flow cytometry further confirmedthe presence of a large B cell population comprising 94% oflymphoid gated events.Theywere kappa light chain restricted(strong expression of light chains) and were CD19 and CD20positive. They aberrantly expressed CD10. They did notexpress CD5 and CD23. FISH was positive for the t(8;14).Overall, the diagnosis was consistent with BL. Diagnosing BLis a challenging process, especially in bodily fluids. However,as shown before [15], the presence of atypical lymphocyteswith characteristic morphologic features and confirmatoryflow cytometry is very helpful in establishing the diagnosis.FISH for t(8;14) is virtually confirmatory of BL.

For staging she underwent a bone marrow biopsy thatwas negative for lymphomatous involvement and a CT of thetorso that showed a decreased, now small pericardial effusion,stable small bilateral pleural effusions, a 2.6 cm lobulatedsoft-tissue lesion in the right atrium, and no evidence oflymphadenopathy. A cardiac MRI revealed on T1- and T2-weighted images a broad base, isointense structuremeasuring3 cm × 1 cm arising from the lateral wall adjacent to theatrioventricular junction of the right atrium (Figure 2). Con-nected to this broad based mass, there was an isodense, cir-cular, well-circumscribed mass measuring 1 cm in diameterprotruding from the broad based mass into the right atrium.Late gadolinium enhancement images showed heterogeneousenhancement of the broad base mass and no enhancementof the circular mass. Her LDH at presentation was normaland an HIV test was negative. A lumbar puncture revealedno evidence of lymphoma cells in the CSF. The patient wastreated with four alternating cycles of modified CODOX-Mand IVAC in combination with rituximab [16] and achieveda complete remission. A follow-up cardiac MRI showed noevidence of the right atrial mass. She remains in remission tothis date, more than 5 years since her initial presentation.

Case Reports in Hematology 3

3. Discussion

Primary cardiac lymphomas are extremely rare but unlikemost other cardiac tumors fast growing and potentially cur-able [3, 17–19].Therefore a high level of suspicion is necessary.Clinical presentation varies and depends on location, size,and growth rate. Dyspnea, lower extremity edema, chest pain,palpitation, and arrhythmia are common presenting symp-toms and sometimes might have systemic (or “B”) symptomssuch as weight loss, anorexia, fatigue, and night sweats. Heartfailure symptoms are due either to intracavity tumor causingobstruction of blood flow through the cardiac chambers orto associated pericardial effusion with cardiac tamponade.BL is faster growing than the other subtypes and patient’spresentations might therefore be more acute and dramatic.

Our patient presented with abdominal and epigastricdiscomfort, which were most likely manifestations of visceralcongestion secondary to elevated right heart filling pressuresassociated with cardiac tamponade.

Certain radiographic characteristics might point towardsa lymphoma [20, 21]. The right atrium is the most commonsite of origin followed by the right ventricle, left ventricle,left atrium, and the interventricular septum. In contrast, themost common type of primary cardiac tumor, myxoma, ismost commonly found in the left atrium, generally attachedto the fossa ovalis. On CT they appear isoattenuating relativeto the myocardium. On CMR they most commonly appearas a large, nodular mass that is isointense or hypointenserelative to myocardium on both T1- and T2-weighted imagesand shows heterogeneous enhancement after administrationof gadolinium.Therefore right atrial location, lack of tumournecrosis, an associated pericardial effusion, and infrequentvalvular involvement should raise the suspicion for lym-phoma. If lymphoma is suspected then a precise pathologicdiagnosis should be the next step, as treatment varies fordifferent subtypes. Our case illustrates the usefulness of flowcytometry in the workup of cardiac tumor-associated peri-cardial effusions immediately after a lymphocytic differential.Cytologicmorphologywithin effusionsmay be deceiving anddoes not provide all the necessary information. Thereforeclinicians who perform pericardiocenteses in patients withsuspected lymphoma of the heart should always order a flowcytometry. Thoracotomy can cause unnecessary treatmentdelay and should be avoided, especially if diagnosis canbe made by less invasive procedures such as percutaneousintracardiac biopsy under ultrasonographic or radiographicguidance, endomyocardial biopsy, or pericardial fluid sam-pling.

Staging procedures should be similar to the other sys-temic lymphomas as heart involvement is usually secondaryrather than the only location. Infection with HIV needs to beruled out as part of the initial workup as extranodal involve-ment in general and heart involvement in particular are morecommon in HIV related lymphomas [22]. Even though thereare not enough data to guide therapy, most patients withcardiac BL have been treated with multiagent chemotherapywith BL’s specific protocols. We chose to treat our patientwith four alternating cycles of CODOX-M and IVAC ratherthan three cycles of CODOX-M [23] as we did not feel

comfortable calling her low-risk even though she formallymet the low-risk criteria used in the LY06 study. We alsoadded rituximab that at least retrospectively is associatedwithbetter survival [16]. Our patient received her first cycle oftreatment on an EKG monitored bed and did not developany arrhythmias. Life threatening arrhythmias however havebeen described after the initiation of chemotherapy and someauthors recommend patientmonitoring in a cardiac intensivecare unit during at least the first cycle [24].

In summary primary cardiac Burkitt lymphoma is anextremely rare and very fast growing tumor of the heart.Suspicion should be high though as cure can be achievedwithmultiagent chemotherapy.

Conflict of Interests

The authors have no conflict of interests to declare.

References

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4 Case Reports in Hematology

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